13095-48-2L-α-Hydroxyglutaric Acid

产品属性：

Cas No.13095-48-2

别名 2(S)-HG, 2(S)-Hydroxyglutaric Acid, L-2-HG, L-2-Hydroxyglutaric Acid

化学名 2S-hydroxy-pentanedioic acid

分子式 C5H8O5

分子量 148.1

溶解度 10mg/mL in PBS, pH 7.2

储存条件 Store at -20°C
General tips  For obtaining a higher solubility , please warm the tube at 37 ℃ and shake it in the ultrasonic bath for a while.
Shipping Condition     Evaluation sample solution : ship with blue ice
All other available size: ship with RT , or blue ice upon request
产品描述：
L-α-Hydroxyglutaric acid (L-2-HG) is an α-hydroxy acid. It is metabolized to 2-oxoglutarate (α-ketoglutarate) by L-2-hydroxyglutarate dehydrogenase, and mutations in this enzyme lead to 2-hydroxyglutaric aciduria, a neurometabolic disorder characterized by increased L-2-HG levels.[1 ][2] L-2-HG is structurally similar to α-ketoglutarate and competitively inhibits α-ketoglutarate-dependent dioxygenases, including several involved in histone lysine and DNA demethylation.[3][4]

Reference:

[1]. Rzem, R., Veiga-da-Cunha, M., No?l, G., et al. A gene encoding a putative FAD-dependent L-2-hydroxyglutarate dehydrogenase is mutated in L-2-hydroxyglutaric aciduria. Proc. Nat. Acad. Sci. USA 101(48), 16849-16854 (2004).

[2]. Struys, E.A., Verhoeven, N.M., Roos, B., et al. Disease-related metabolites in culture medium of fibroblasts from patients with D-2-hydroxyglutaric aciduria, L-2-hydroxyglutaric aciduria, and combined D/L-2-hydroxyglutaric aciduria. Clin. Chem. 49(7), 1133-1138 (2003).

[3]. Chowdhury, R., Yeoh, K.K., Tian, Y.M., et al. The oncometabolite 2-hydroxyglutarate inhibits histone lysine demethylases. EMBO Rep. 12(5), 463-469 (2011).

[4]. Xu, W., Yang, H., Liu, Y., et al. Oncometabolite 2-hydroxyglutarate is a competitive inhibitor of α-ketoglutarate-dependent dioxygenases. Cancer Cell 19(1), 17-30 (2011).